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Abnormal involuntary movement (AIM) are usually classified into hypokinesia and hyperkinesia group. Hyperkinesia-AIM includes myoclonus, chorea, ballism, dystonia, athetosis, and more. Of these, dystonia, myoclonus, and chorea are frequent movement disorders. From a neurophysiological point of view, the mechanism of motor control by the basal ganglia is thought to consist of three pathways: hyperdirect, direct, and indirect. Hyperkinetic-AIMs are likely caused by the dysfunction of any of these three pathways, leading to malfunction in either presurround inhibition, initiation of motor performance, or postsurround inhibition. These dysfunctions are assumed to stem from regions, such as the cerebral cortex, white matter, basal ganglia, brainstem, and cerebellum. Drug therapies that consider the pathogenesis mechanism are desirable. Here, we presented an overview of treatment methods for hyperkinetic-AIMs.

Background: Dystonia is characterized by sustained or intermittent muscle contractions, leading to abnormal posturing and twisting movements. In pediatric patients, dystonia often negatively influences quality of life. Pharmacological treatment for dystonia is often inadequate and causes adverse effects. Deep brain stimulation (DBS) appears to be a valid therapeutic option for pharmacoresistant dystonia in children., Methods: To illustrate the current clinical practice, we hereby describe two pediatric cases of monogenetic movement disorders presenting with dystonia and treated with DBS. We provide a literature review of similar previously described cases and on different clinical aspects of DBS in pediatric dystonia., Results: The first patient, a 6-year-old girl with severe dystonia, chorea, and myoclonus due to an ADCY5 gene mutation, received DBS in an elective setting. The second patient, an 8-year-old boy with GNAO1 -related dystonia and chorea, underwent emergency DBS due to a pharmacoresistant status dystonicus. A significant amelioration of motor symptoms (65% on the Burke-Fahn-Marsden Dystonia Rating Scale) was observed postoperatively in the first patient and her personal therapeutic goals were achieved. DBS was previously reported in five patients with ADCY5 -related movement disorders, of which three showed objective improvement. Emergency DBS in our second patient resulted in the successful termination of his GNAO1 -related status dystonicus, this being the eighth case reported in the literature., Conclusion: DBS can be effective in monogenetic pediatric dystonia and should be considered early in the disease course. To better evaluate the effects of DBS on patients' functioning, patient-centered therapeutic goals should be discussed in a multidisciplinary approach., Competing Interests: M.B. was awarded the TKI-PPP grant in 2021. A.I.B. is the chair of the board of the Dutch Academy of Childhood Disability. J.M.D. was awarded the Netherlands Organization for Health Research and Development grant and a Medtronic grant. J.M.D. is the president of the Netherlands workgroup for Movement Disorders. P.R.S. was awarded consulting fees from Boston Scientific, Medtronic, Elekta and lecture fees from Boston Scientific and Elekta. P.R.S. is the treasurer of the European Society for Stereotactic and Functional Neurosurgery, an associate editor of the Journal of Parkinson's Disease and is in the advisory board of Boston Scientific and Insightec., (The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).)

Movement disorders comprise a heterogeneous and complex group of neurological disorders that increase (hyperkinetic) or decrease (hypokinetic) the speed or amplitude of movements, or disrupt their coordinated sequencing. In this article, we describe three instructive cases, exemplifying classic movement disorders, namely dystonia, chorea, and ataxia. We highlight the diagnostic approach based on clinical clues, syndromic reasoning, evaluation, and management recommendations. Each case ends with key messages for the clinicians., Competing Interests: None declared., (Thieme. All rights reserved.)

Dystonia is a clinical sign and main feature of many movement disorders in humans as well as veterinary species. It is characterized by sustained or intermittent involuntary muscle contractions causing abnormal (often repetitive) movements, postures, or both. This review discusses the terminology and definition of dystonia, its phenomenology, and its pathophysiology, and provides considerations regarding the diagnosis and treatment of dystonia in dogs and cats. In addition, currently recognized or reported disorders in dogs and cats in which dystonia is a particular or main feature are discussed and comparisons are made between disorders featuring dystonia in humans and animals. We suggest that when describing the phenomenology of dogs and cats with dystonia, if possible the following should be included: activity being performed at onset (e.g., resting or running or exercise-induced), body distribution, duration, responsiveness (subjective), severity, temporal pattern (i.e., paroxysmal or persistent, severity at onset and at later stages), presence or absence of autonomic signs (e.g., salivation), presence or absence of preceding signs (e.g., restlessness), presence or absence of signs after dystonia subsides (e.g., sleepiness), coexistence of other movement disorders, any other neurological manifestations, and possible links to administered medications, intoxications or other associated factors. We also suggest that dystonia be classified based on its etiology as either structural genetic, suspected genetic, reactive, or unknown., (© 2022 The Authors. Journal of Veterinary Internal Medicine published by Wiley Periodicals LLC on behalf of American College of Veterinary Internal Medicine.)

Purpose of Review: This article discusses the most recent findings regarding the diagnosis, classification, and management of genetic and idiopathic dystonia., Recent Findings: A new approach to classifying dystonia has been created with the aim to increase the recognition and diagnosis of dystonia. Molecular biology and genetic studies have identified several genes and biological pathways involved in dystonia., Summary: Dystonia is a common movement disorder involving abnormal, often twisting, postures and is a challenging condition to diagnose. The pathophysiology of dystonia involves abnormalities in brain motor networks in the context of genetic factors. Dystonia has genetic, idiopathic, and acquired forms, with a wide phenotypic spectrum, and is a common feature in complex neurologic disorders. Dystonia can be isolated or combined with another movement disorder and may be focal, segmental, multifocal, or generalized in distribution, with some forms only occurring during the performance of specific tasks (task-specific dystonia). Dystonia is classified by clinical characteristics and presumed etiology. The management of dystonia involves accurate diagnosis, followed by treatment with botulinum toxin injections, oral medications, and surgical therapies (mainly deep brain stimulation), as well as pathogenesis-directed treatments, including the prospect of disease-modifying or gene therapies., (Copyright © 2022 American Academy of Neurology.)

Deep brain stimulation (DBS) is an established intervention for use in pediatric movement disorders, especially dystonia. Although multiple publications have provided guidelines for deep brain stimulation patient selection and programming in adults, there are no evidence-based or consensus statements published for pediatrics. The result is lack of standardized care and underutilization of this effective treatment. To this end, we assembled a focus group of 13 pediatric movement disorder specialists and 1 neurosurgeon experienced in pediatric deep brain stimulation to review recent literature and current practices and propose a standardized approach to candidate selection, implantation target site selection, and programming algorithms. For pediatric dystonia, we provide algorithms for (1) programming for initial session and follow-up sessions, and (2) troubleshooting side effects encountered during programming. We discuss common side effects, how they present, and recommendations for management. This topical review serves as a resource for movement disorders specialists interested in using deep brain stimulation for pediatric dystonia.

Intracerebral recordings from movement disorders patients undergoing deep brain stimulation have allowed the identification of pathophysiological patterns in oscillatory activity that correlate with symptom severity. Changes in oscillatory synchrony occur within and across brain areas, matching the classification of movement disorders as network disorders. However, the underlying mechanisms of oscillatory changes are difficult to assess in patients, as experimental interventions are technically limited and ethically problematic. This is why animal models play an important role in neurophysiological research of movement disorders. In this review, we highlight the contributions of translational research to the mechanistic understanding of pathological changes in oscillatory activity, with a focus on parkinsonism and dystonia, while addressing the limitations of current findings and proposing possible future directions., (Copyright © 2022 The Authors. Published by Elsevier Inc. All rights reserved.)

Purpose of Review: Deep brain stimulation (DBS) is an established treatment in several movement disorders, including Parkinson's disease, dystonia, tremor, and Tourette syndrome. In this review, we will review and discuss the most recent findings including but not limited to clinical evidence., Recent Findings: New DBS technologies include novel hardware design (electrodes, cables, implanted pulse generators) enabling new stimulation patterns and adaptive DBS which delivers potential stimulation tailored to moment-to-moment changes in the patient's condition. Better understanding of movement disorders pathophysiology and functional anatomy has been pivotal for studying the effects of DBS on the mesencephalic locomotor region, the nucleus basalis of Meynert, the substantia nigra, and the spinal cord. Eventually, neurosurgical practice has improved with more accurate target visualization or combined targeting. A rising research domain emphasizes bridging neuromodulation and neuroprotection. Recent advances in DBS therapy bring more possibilities to effectively treat people with movement disorders. Future research would focus on improving adaptive DBS, leading more clinical trials on novel targets, and exploring neuromodulation effects on neuroprotection., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Background: Further reports are required to describe the outcome of truncal dystonia treated by bilateral pallidal stimulation (globus pallidus interna deep brain stimulation [GPi-DBS]), owing to the small number of reports and clinical variability and complexity of truncal dystonia. Retrospectively, we report our experience of treating three patients with idiopathic generalized dystonia, with predominant mobile truncal dystonia by bilateral GPi-DBS., Methods: Three patients with idiopathic generalized dystonia underwent bilateral GPi-DBS. One patient had adult-onset dystonia, while two patients had childhood-onset dystonia. All patients had predominant mobile truncal dystonia of mixed abnormal postures (camptocormia and lateral tilt), while one patient had also truncal twist. Patients were assessed pre- and post-GPi-DBS using the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) and Dystonia Disability Scale (DDS)., Results: The three patients showed marked improvement of global (94.78%, 92.4% and 80.95%) and truncal BFMDRS (all abnormal postures) (87.5%, 93.75% and 87.5%) and DDS (95.84% and 50%), using high amplitude monopolar settings, with a dramatic improvement of the mobile component. Improvement was persistent for 1.5, 3 and 6 years., Conclusion: Bilateral GPi-DBS improves markedly the mobile truncal dystonia and associated abnormal postures in patients with adult and childhood-onset idiopathic generalized dystonia. Improvement was persistent for up to 6 years.

Background: Japanese encephalitis (JE) is a potentially fatal viral infection with a wide range of manifestations and can also present with a variety of movement disorders (MD) including dystonia. Dystonic features in JE are uncommon. Here, we have tried to summarize the clinical features and management of dystonia among JE patients with a comprehensive literature search., Methods: Various databases, including PubMed, Embase, and Google Scholar, were searched against the predefined criteria using suitable keywords combination and boolean operations. Relevant information from observational and case studies was extracted according to the author, dystonic features, radiological changes in the brain scans, treatment options, and outcome wherever provided., Result: We identified 19 studies with a total of 1547 JE patients, the diagnosis of which was confirmed by IgM detection in serum and/or cerebrospinal fluid in the majority of the patients (88.62%). 234 (15.13%) of JE patients had dystonia with several types of focal dystonia being present in 131 (55.98%) either alone or in combination. Neuroimaging showed predominant involvement of thalami, basal ganglia, and brainstem. Oral medications including anticholinergics, GABA agonists, and benzodiazepines followed by botulinum toxin were the most common treatment modalities., Conclusion: Dystonia can be a disabling consequence of JE, and various available medical therapies can significantly improve the quality of life. Owing to insufficient studies on the assessment of dystonia associated with JE, longitudinal studies with a larger number of patients are warranted to further clarify the clinical course, treatment, and outcome of dystonia., (© 2022 The Authors. Brain and Behavior published by Wiley Periodicals LLC.)

Sex and gender-based differences in epidemiology, clinical features and therapeutical responses are emerging in several movement disorders, even though they are still not widely recognized. In this chapter, we summarize the most relevant evidence concerning these differences in Parkinson's disease, essential tremor, dystonia and chorea. Indeed, both sex-related biological (hormonal levels fluctuations) and gender-related variables (socio-cultural and environmental factors) may differently impact symptoms manifestation and severity, phenotype and disease progression of movement disorders on men and women. Moreover, sex differences in treatment responses should be taken into account in any therapeutical planning. Physicians need to be aware of these major differences between men and women that will eventually have a major impact on better tailoring prevention, treatment, or even delaying progression of the most common movement disorders., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Background: Task-specific dystonia (TSD) is a form of focal dystonia that occurs in the context of the performance of selective, highly skilled, often repetitive, motor activity. TSD may be apparent during certain tasks such as writing, playing musical instruments, or other activities requiring fine motor control, but may also occur during certain sports, and maybe detrimental to professional athletes' careers. Therefore, sports physicians and movement disorder neurologists need to be aware of the presentation and phenomenology of sports-related dystonia (SRD), the topic of this review., Methods: A broad PubMed search using a wide range of keywords and combinations was done in October 2021 to identify suitable articles for this review., Results: Most of the publications are on yips in golfers and on runners' dystonia. Other sports in which SRD has been reported are ice skating, tennis, table tennis, pistol shooting, petanque, baseball, and billiards., Discussion: Yips, which may affect up to half of the golfers and rarely athletes in other sports (e.g., baseball, cricket, basketball, speed skating, gymnastics) seems to be a multi-factorial form of TSD that is particularly troublesome in highly skilled professional golfers. Runners' dystonia, affecting the foot, leg, and hip (in decreasing order), may evolve into more generalized and less specific dystonia. The pathophysiologic mechanisms of SRD are not well understood. Botulinum toxin has been reported to alleviate dystonia in golfers', runners', and other forms of SRD. Future studies should utilize neurophysiologic, imaging, and other techniques to elucidate mechanisms of this underrecognized group of movement disorders., Competing Interests: The authors have no competing interests to declare., (Copyright: © 2021 The Author(s).)

Context: Movement disorders represent a common presentation in pediatrics and are often a source of clinical and diagnostic dilemmas. In this review, we provide an overview of common causes along with simplified clinical approach and management options for major movement disorders., Sources: This narrative review is based on contemporary evidence and personal experience. Medline was searched for recent advances, current understanding and consensus on classification, clinical features, diagnosis and treatment., Results: Movement disorders are classified as hyperkinetic and hypokinetic disorders, the latter being rare in childhood. The hyperkinetic disorders include dystonia, chorea, athetosis, tics and tremor, stereotypies, myoclonus, startle syndromes and functional disorders. Some movement disorders can be benign and developmental. A large proportion of conditions are genetic in origin with a guarded prognosis. Some of the conditions may be post-infectious, immune-mediated or drug induced. Multiple types of movement disorders are present in many conditions. The age at onset, type and distribution of abnormal movements and presence of associated neurological and systemic features help in narrowing the differential diagnosis. The pharmacotherapy of movement disorders is complex and evolving., Conclusion: A synopsis of movement disorders presenting in pediatric age has been provided, incorporating the latest evidence. A simplified approach for clinical diagnosis has been developed for dystonia and chorea.

Dystonia is one of the most common pediatric movement disorders and can have a profound impact on the lives of children and their caregivers. Response to pharmacologic treatment is often unsatisfactory. Deep brain stimulation (DBS) has emerged as a promising treatment option for children with medically refractory dystonia. In this review we highlight the relevant literature related to DBS for pediatric dystonia, with emphasis on the background, indications, prognostic factors, challenges, and future directions of pediatric DBS., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Stereotactic lesioning of the bilateral globus pallidus (GPi) was one of the first surgical treatments for medication-refractory dystonia but has largely been abandoned in clinical practice after the introduction of deep brain stimulation (DBS). However, some patients with dystonia are not eligible for DBS. Therefore, we reviewed the efficacy, safety, and sustainability of bilateral pallidotomy by conducting a systematic review of individual patient data (IPD). Guidelines of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses and IPD were followed. In May 2020, Medline, Embase, Web of Science, and Cochrane Library were searched for studies reporting on outcome of bilateral pallidotomy for dystonia. If available, IPD were collected. In this systematic review, 100 patients from 33 articles were evaluated. Adverse events were reported in 20 patients (20%), of which 8 were permanent (8%). Pre-and postoperative Burke-Fahn-Marsden Dystonia Rating Movement Scale scores were available for 53 patients. A clinically relevant improvement (>20%) of this score was found in 42 of 53 patients (79%). Twenty-five patients with status dystonicus (SD) were described. In all but 2 the SD resolved after bilateral pallidotomy. Seven patients experienced a relapse of SD. Median-reported follow-up was 12 months (n = 83; range: 2-180 months). Based on the current literature, bilateral pallidotomy is an effective and relatively safe procedure for certain types of dystonia, particularly in medication-refractory SD. Although due to publication bias the underreporting of negative outcomes is very likely, bilateral pallidotomy is a reasonable alternative to DBS in selected dystonia patients. © 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society., (© 2020 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.)

Deep brain stimulation (DBS) is the most commonly used surgical treatment for drug-refractory movement disorders such as tremor and dystonia. Appropriate patient selection along with target selection is important to ensure optimal outcome without complications. This review summarizes the recent literature regarding the mechanism of action, indications, outcome, and complications of DBS in tremor and dystonia. A comparison with other modalities of surgical interventions is discussed along with a note of the recent advances in technology. Future research needs to be directed to understand the underlying etiopathogenesis of the disease and the way in which DBS modulates the intracranial abnormal networks., Competing Interests: None

Deep brain stimulation is the most advanced and effective neuromodulation therapy for Parkinson disease, essential tremor, and generalized dystonia. This article discusses how imaging improves surgical techniques and outcomes and widens possibilities in translational neuroscience in Parkinson disease, essential tremor, generalized dystonia, and epilepsy. In movement disorders diffusion tensor imaging allows anatomic segment of cortical areas and different functional subregions within deep-seated targets to understand the side effects of stimulation and gain more data to describe the therapeutic mechanism of action. The introduction of visualization of white matter tracks increases the safety of neurosurgical techniques in functional neurosurgery and neuro-oncology., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: The introduction of intracranial air (ICA) during deep brain stimulation (DBS) surgery is thought to have a negative influence on targeting and clinical outcomes., Objective: To investigate ICA volumes following surgery and other patient-specific factors as potential variables influencing translocation of the DBS electrode and proximal lead bowing., Methods: High-resolution postoperative computed tomography scans (≤1.0 mm resolution in all directions) within 24 h following DBS surgery and 4-6 weeks of follow-up were acquired. A total of 50 DBS leads in 33 patients were available for analysis. DBS leads included Abbott/St. Jude Medical InfinityTM, Boston Scientific VerciseTM, and Medtronic 3389TM., Results: Both ICA volume and anatomical target were significantly associated with measures of DBS electrode translocation. ICA volume and DBS lead model were found to be significant predictors of proximal lead bowing. Measures of proximal lead bowing and translocation along the electrode trajectory for the Medtronic 3389TM DBS lead were significantly larger than measures for the Abbott/St. Jude Medical InfinityTM and Boston Scientific VerciseTM DBS leads., Conclusion: The association between ICA volume and translocation of the DBS electrode is small in magnitude and not clinically relevant for DBS cases within a normal range of postoperative subdural air volumes. Differences in proximal lead bowing observed between DBS leads may reflect hardware engineering subtleties in the construction of DBS lead models., (© 2020 S. Karger AG, Basel.)

Objective: This study investigates the association between pallidal low-frequency activity and motor sign severity in dystonia after chronic deep brain stimulation for several months., Methods: Local field potentials were recorded in 9 dystonia patients at 5 timepoints (T1-T5) during an OFF-stimulation period of 5 to 7 hours in parallel with clinical assessment using Burke-Fahn-Marsden Dystonia Rating Scale. A linear mixed effects model was used to investigate the potential association of motor signs with local field potential activity in the low frequency (3-12 Hz) and beta range (13-30 Hz)., Results: A significant association of Burke-Fahn-Marsden Dystonia Rating Scale scores with low-frequency activity (3-12 Hz; b = 4.4; standard error = 1.5, degrees of freedom = 43, P = 0.006, 95% confidence interval, 1.3-7.5), but not beta activity (13-30 Hz) was revealed within participants across timepoints., Conclusion: Low-frequency activity is associated with dystonic motor sign severity, even months after chronic deep brain stimulation. Our findings corroborate the pathophysiological role of low-frequency activity in dystonia and highlight the potential utility as a biomarker for adaptive neuromodulation. © 2019 International Parkinson and Movement Disorder Society., (© 2019 International Parkinson and Movement Disorder Society.)

Deep brain stimulation (DBS) is a treatment which uses high-frequency electric stimulation to suppress pathological brain activity. DBS has been applied for over 30 years now, particularly in patients with severe movement disorders, such as Parkinson's disease, dystonia and tremor. Although there is clearly scientific evidence for the effectiveness of DBS in these three movement disorders, the effect size of the treatment remains limited. Furthermore, DBS is not curative and can only be applied in a select subset of patients. In this article, we discuss the key indications and contraindications for DBS, and the outcomes achieved when it is applied in the aforementioned movement disorders. We discuss the most notable controversies and new developments in the field of deep brain stimulation, in order to offer referrers and fellow healthcare professionals an accessible introduction to this mode of therapy.

Neurology still remains one of the most underserved specialties of medicine in Pakistan with roughly one neurologist per million people. Movement disorders (MD) are neurological problems that interfere with patient's motor abilities and diagnosis is typically clinical. In this review, we describe a practical approach to common MD emergencies that may be encountered by a non-neurologist physician, emphasizing on formulating a working diagnosis and their immediate management. Movement disorder emergencies can be classified based on MD phenomenology and we will provide a brief overview of dystonia including acute dystonic reaction, PAID syndrome and dystonic storm; chorea, myoclonus including serotonin syndrome and startle disease; and rigidity including neuroleptic malignant syndrome and malignant hyperthermia.

Background: A large number of methods have been described that use transcranial magnetic stimulation to probe the physiology of the human motor cortex. Since the 1990s, hundreds of papers have used them to investigate neurophysiological signatures of different types of movement disorders. However, in recent years there has been increasing recognition of the interindividual variability of these measures and a focus on estimating their reliability and reproducibility. Although this work has been carried out in healthy individuals, it is highly relevant to movement disorders because it may impact the validity of some accepted ("canonical") neurophysiological biomarkers. The aim of this review is to reexamine the diagnostic usefulness of transcranial magnetic stimulation methods in movement disorders in the context of present knowledge of methodological variability., Methods: We conducted a search of the PubMed database for research and review articles on transcranial magnetic stimulation and its diagnostic utility in movement disorders (specifically Parkinson's disease and atypical parkinsonism, dystonia, Tourette syndrome and other chronic tic disorders, Huntington's disease, and essential tremor). We highlighted contradictions in the literature and common misconceptions with the aim of providing a clearer picture of the reliability of these measures in differential diagnosis of movement disorders., Conclusion: Although there is no doubt that these studies have provided useful insight into the pathophysiology of movement disorders, there is a clear disagreement among many studies that questions the validity of some of the so called "canonical" findings as diagnostic markers. However, useful findings remain and even those with higher variability can be used to support clinical diagnosis in selected cases. © 2019 International Parkinson and Movement Disorder Society., (© 2019 International Parkinson and Movement Disorder Society.)

Deep Brain Stimulation (DBS) was introduced into clinical practice nearly four decades ago and is currently the standard of care for patients with Parkinson's disease experiencing motor complications. Apart from this, it has several other established and emerging applications in movement disorders. The exact mechanisms by which DBS provides relief in movement disorders are still unclear; disruption of pathological neuronal synchrony and abnormal information flow through the neuronal circuits involved, are the most likely underlying mechanisms. DBS has been established to be a relatively safe procedure if patients are carefully selected and followed up by experienced multidisciplinary teams. Alternatives to the traditional stereotactic frame based techniques of lead implantation are emerging, and these, along with the other recent technological advances, are likely to extend the availability of this therapy to an increasing number of patients in the future., Competing Interests: There are no conflicts of interest.

It has been three decades since the first application of deep brain stimulation (DBS) for tremors was described by Benabid. Over the years, the indications for the performance of DBS have been expanding. There are now more than 1,50,000 patients around the world who have undergone DBS for various disorders. The main appeal of DBS is in its reversibility and titratability. Though the initial interest in DBS was for pain, the main indications for DBS have been movement disorders. Despite its wide appeal and "perceived" advantage, United States Food and Drug Administration, the nodal agency for approving therapies, has been cautious and guarded in providing approvals. Only two indications, i.e., Parkinson's disease and tremors, have been approved; the two other indications, i.e., dystonia and obsessive compulsive disorder (OCD), have been granted exemption under the humanitarian device usage. However, the European community has been more liberal and several of these indications have CE (Conformite Europeene) approval. Most of them will be reviewed in this article. There have been numerous indications for which DBS has been applied, which in turn has helped to change the lives of several patients. Unfortunately, due to the paucity of the number of procedures performed and the inherent difficulty in conducting "surgical" double blind randomized trials, Class 1 or Class 2 evidence for several of these indications is lacking. Hence, it is advisable that one does not embark on using each and any target for each and any indication without having the understanding or the team backup. It is cautionary that most of these therapies should be conducted in an institutional setting with an ethics and scientific committee backup and ably assisted by an experienced team., Competing Interests: There are no conflicts of interest.

Parkinson's disease (PD) and dystonia are common indications for the deep brain stimulation (DBS) procedure. It is very important to be diligent about target localization and execution of the procedure. The single most important predictor of a good postoperative outcome is proper patient selection. The various steps of performing DBS include taking a preoperative non - stereotactic MRI, stereotactic frame fixation, fusion of MRI with stereotactic CT scan images, planning of the target and trajectory, lead placement at target through the planned trajectory, implantation of pulse generator/ battery and programming of the implanted device. Utmost care and precision are required to execute the procedure, which decide the final outcome of the surgical procedure., Competing Interests: There are no conflicts of interest.

Purpose of Review: To demonstrate how neurophysiological tools have advanced our understanding of the pathophysiology of paediatric movement disorders, and of neuroplasticity in the developing brain., Recent Findings: Delineation of corticospinal tract connectivity using transcranial magnetic stimulation (TMS) is being investigated as a potential biomarker for response to therapy. TMS measures of cortical excitability and neuroplasticity are also being used to investigate the effects of therapy, demonstrating neuroplastic changes that relate to functional improvements. Analyses of evoked potentials and event-related changes in the electroencephalogaphy spectral activity provide growing evidence for the important role of aberrant sensory processing in the pathophysiology of many different movement disorders. Neurophysiological findings demonstrate that children with clinically similar phenotypes may have differing underlying pathophysiology, which in turn may explain differential response to therapy. Neurophysiological parameters can act as biomarkers, providing a means to stratify individuals, and are well suited to provide biofeedback. They therefore have enormous potential to facilitate improvements to therapy., Summary: Although currently a small field, the role of neurophysiology in paediatric movement disorders is poised to expand, both fuelled by and contributing to the rapidly growing fields of neuro-rehabilitation and neuromodulation and the move towards a more individualized therapeutic approach.

The deep brain stimulation (DBS) in movement disorders is well established and in many aspects evidence-based procedure. The treatment indications are very heterogeneous and very specific in their course and therapy. The deep brain stimulation plays very important, but usually not the central role in this conditions. The success in the application of DBS is essentially associated with the correct, appropriate and timely indication of the therapy in the course of these diseases. Thanks to the good standardization of the DBS procedure and sufficient published data, the recommendations for indication, diagnosis and operative procedures can be generated. The following article attempts to summarize the most important decision-making criteria and current therapy standards in this fairly comprehensive subject and to present them in close proximity to practice., Competing Interests: Interessenkonflikt: Die Autoren geben an, dass kein Interessenkonflikt vorliegt., (Georg Thieme Verlag KG Stuttgart · New York.)

Stroke may be associated with different types of movement disorders, such as hyperkinetic syndromes (hemichorea-hemiballism, unilateral asterixis, limb-shaking, dystonia, tremor, myoclonus) and hypokinetic syndromes (especially vascular parkinsonism). However, movement disorders are rare and transient in acute stroke and, as a permanent consequence, are more often delayed. While ischemic and hemorrhagic strokes can happen at any level of the frontal-subcortical motor system, they can be explained most of the time by a dysfunction in the basal ganglia motor circuit. However, only brain MRI allows the involved structure(s) to be precisely located, and each syndrome is specific to the type of lesion. Treatment is above all symptomatic. Only limb-shaking syndrome requires urgent surgical treatment because of the low-perfusion hemodynamic state. The functional prognosis depends on the type of movement disorder., (Copyright © 2016. Published by Elsevier Masson SAS.)

Deep brain stimulation (DBS) is highly effective for both hypo- and hyperkinetic movement disorders of basal ganglia origin. The clinical use of DBS is, in part, empiric, based on the experience with prior surgical ablative therapies for these disorders, and, in part, driven by scientific discoveries made decades ago. In this review, we consider anatomical and functional concepts of the basal ganglia relevant to our understanding of DBS mechanisms, as well as our current understanding of the pathophysiology of two of the most commonly DBS-treated conditions, Parkinson's disease and dystonia. Finally, we discuss the proposed mechanism(s) of action of DBS in restoring function in patients with movement disorders. The signs and symptoms of the various disorders appear to result from signature disordered activity in the basal ganglia output, which disrupts the activity in thalamocortical and brainstem networks. The available evidence suggests that the effects of DBS are strongly dependent on targeting sensorimotor portions of specific nodes of the basal ganglia-thalamocortical motor circuit, that is, the subthalamic nucleus and the internal segment of the globus pallidus. There is little evidence to suggest that DBS in patients with movement disorders restores normal basal ganglia functions (e.g., their role in movement or reinforcement learning). Instead, it appears that high-frequency DBS replaces the abnormal basal ganglia output with a more tolerable pattern, which helps to restore the functionality of downstream networks.

Movement disorders such as Parkinson's disease (PD), essential tremor (ET) and dystonia can benefit from deep brain stimulation (DBS). DBS is considered when symptoms are disabling despite optimal medical therapy. Contraindications include dementia, uncontrolled psychiatric disease and/or comorbid conditions with potential for evolution. Targets are the subthalamic nucleus for PD, the ventral intermediate nucleus for ET and the globus pallidus internus for dystonia. The beneficial effet of DBS has been well documented for symptom control. Optimal target localization of the electrodes reduces the occurrence of side-effects. Stimulation-induced adverse effects can usually be abolished by turning the stimulation off, changing the active contact or other stimulation parameters.

Epidural spinal cord stimulation (SCS) is currently proposed to treat intractable neuropathic pain. Since the 1970s, isolated cases and small cohorts of patients suffering from dystonia, tremor, painful leg and moving toes (PLMT), or Parkinson’s disease were also treated with SCS in the context of exploratory clinical studies. Despite the safety profile of SCS observed in these various types of movement disorders, the degree of improvement of abnormal movements following SCS has been heterogeneous among patients and across centers in open-label trials, stressing the need for larger, randomized, double-blind studies. This article provides a comprehensive review of both experimental and clinical studies of SCS application in movement disorders.

Deep brain stimulation (DBS) is an implanted electrical device that modulates specific targets in the brain resulting in symptomatic improvement in a particular neurologic disease, most commonly a movement disorder. It is preferred over previously used lesioning procedures due to its reversibility, adjustability, and ability to be used bilaterally with a good safety profile. Risks of DBS include intracranial bleeding, infection, malposition, and hardware issues, such migration, disconnection, or malfunction, but the risk of each of these complications is low--generally ≤ 5% at experienced, large-volume centers. It has been used widely in essential tremor, Parkinson's disease, and dystonia when medical treatment becomes ineffective, intolerable owing to side effects, or causes motor complications. Brain targets implanted include the thalamus (most commonly for essential tremor), subthalamic nucleus (most commonly for Parkinson's disease), and globus pallidus (Parkinson's disease and dystonia), although new targets are currently being explored. Future developments include brain electrodes that can steer current directionally and systems capable of "closed loop" stimulation, with systems that can record and interpret regional brain activity and modify stimulation parameters in a clinically meaningful way. New, image-guided implantation techniques may have advantages over traditional DBS surgery.

Background: Deep brain stimulation (DBS) is a surgical treatment involving the implantation of a brain lead connected to a chest-based neurostimulator similar to a cardiac pacemaker. The device can be programmed to deliver electrical impulses to neuromodulate abnormal brain circuitry in disorders such as Parkinson's disease (PD), essential tremor (ET), and dystonia. As the number of patients receiving DBS surgery increases, it will be important for primary care doctors to identify reasonable DBS candidates for referral to an experienced center., Objective: To provide primary care physicians with a rationale and also to provide clinically useful pearls for referral of potential DBS candidates., Methods: A complete PubMed review of the literature., Results: This review will be focused on PD and ET and will address the following issues: what are the common motor and nonmotor symptoms? What is the evidence supporting the use of DBS in PD and ET? What is the importance of a multi- or interdisciplinary DBS team for patient selection? What can be done to improve success in identifying and referring potential DBS candidates?, Conclusion: DBS is a highly effective therapy for select candidates with PD and ET. The most important factor influencing DBS outcome is proper patient selection. It will be critical as DBS continues to be more commonly employed for primary care doctors to select candidates from their practices as appropriate referrals to specialized centers.

Background: Deep brain stimulation (DBS) for the treatment of movement disorders has provided researchers with an opportunity to record electrical oscillatory activity from electrodes implanted in deep brain structures. Extracellular activity recorded from a population of neurons, termed local field potentials (LFPs), has shed light on the pathophysiology of movement disorders and holds the potential to lead to refinement in existing treatments., Objective: This paper reviews the clinical significance of LFPs recorded from macroelectrodes implanted in basal ganglia and thalamic targets for the treatment of Parkinson's disease, essential tremor and dystonia., Methods: Neural population dynamics and subthreshold events, which are undetectable by single-unit recordings, can be examined with frequency band analysis of LFPs (frequency range: 1-250 Hz)., Results: Of clinical relevance, reliable correlations between motor symptoms and components of the LFP power spectrum suggest that LFPs may serve as a biomarker for movement disorders. In particular, Parkinson's rigidity has been shown to correlate with the power of beta oscillations (13-30 Hz), and essential tremor coheres with oscillations of 8-27 Hz. Furthermore, evidence indicates that the optimal contacts for DBS programming can be predicted from the anatomic location of beta and gamma bands (48-200 Hz)., Conclusion: LFP analysis has implications for improved electrode targeting and the development of a real-time, individualized, 'closed-loop' stimulation system., (2014 S. Karger AG, Basel.)

Background: Childhood dystonia can severely impact upper limb function. Deep brain stimulation (DBS) has been shown to be effective in reducing dystonic symptoms in childhood. Functional recovery following DBS is however not well understood., Aims: To explore changes in upper limb function following DBS in paediatric dystonia., Methods: Upper limb outcomes, using the Melbourne Assessment of Unilateral Upper Limb Function, are reported in 20 cases of childhood dystonia (unilateral n = 1, four limb n = 19) at 6 and 12 months following DBS., Results: Improvement in at least in one upper limb was seen in the majority of cases (n = 17, 85%) at 12 months following DBS. Deterioration of scores in both upper limbs was seen in 3 children with progressive disorders. Grouping the children aetiologically, a significant improvement in the dominant hand was obtained for the primary dystonia/dystonia-plus group at both six (p = 0.018) and twelve months (p = 0.012). In secondary dystonia due to a static disorder, improvement was also seen at 6 (p = 0.043) and 12 months (p = 0.046) in the non-dominant hand. No significant change was found in the group of children with progressive disorders., Conclusions: DBS has the potential to alter upper limb function in children with primary and secondary dystonia. The dominant hand improved most in children with primary dystonias, with greater improvement in the non-dominant hand in secondary-static cases., (Copyright © 2013 European Paediatric Neurology Society. Published by Elsevier Ltd. All rights reserved.)

Deep brain stimulation (DBS) has emerged as an effective neurosurgical tool to treat a range of conditions. Its use in movement disorders such as Parkinson's disease, tremor and dystonia is now well established and has been approved by the National Institute of Clinical Excellence (NICE). The NICE does, however, emphasise the need for a multidisciplinary team to manage these patients. Such a team is traditionally composed of neurologists, neurosurgeons and neuropsychologists. Neuropsychiatrists, however, are increasingly recognised as essential members given many psychiatric considerations that may arise in patients undergoing DBS. Patient selection, assessment of competence to consent and treatment of postoperative psychiatric disease are just a few areas where neuropsychiatric input is invaluable. Partly driven by this close team working and partly based on the early history of DBS for psychiatric disorders, there is increasing interest in re-exploring the potential of neurosurgery to treat patients with psychiatric disease, such as depression and obsessive-compulsive disorder. Although the clinical experience and evidence with DBS in this group of patients are steadily increasing, many questions remain unanswered. Yet, the characteristics of optimal surgical candidates, the best choice of DBS target, the most effective stimulating parameters and the extent of postoperative improvement are not clear for most psychiatric conditions. Further research is therefore required to define how DBS can be best utilised to improve the quality of life of patients with psychiatric disease.

A number of genetic causes of movement disorders including Parkinson disease, dystonia, restless legs syndrome or essential tremor have been elucidated in recent years. This process was accelerated by novel technologies including genome-wide association studies (GWAS) and next generation sequencing (NGS). Although monogenic forms are overall rare, they provide a unique opportunity to investigate mutation carriers who are still in the presymptomatic phase. As these subjects present individuals at risk to develop the disease, they have been included in longitudinal studies to unravel disease mechanisms and elucidate novel therapeutic targets. In addition, cell culture and animal studies have been performed to functionally characterize proteins mutated in different movement disorders to provide further insight into disturbed cellular pathways. In this article, we summarize known monogenic forms and the associated phenotype as well as genetic risk factors and review the function of relevant genes and proteins.

Deep brain stimulation (DBS) has been widely performed for various medically refractory movement disorders. For Parkinson disease (PD), target of DBS is subthalamic nucleus (STN) or globus pallidus internus (GPi). There are some evidences to demonstrate its effectiveness on motor function and QOL. DBS effectively improves levodopa-responsive motor symptoms, and significantly reduces dyskinesia and motor fluctuations. STN DBS particularly decreases the need for dopaminergic medication. A combination of medical treatment and DBS would provide longer relief of motor symptoms than medical treatment only. Currently new evidence supporting early introduction of DBS for better future outcome is published. DBS of Vim thalamus has been indicated for intractable tremor with various etiologies. Recently posterior subthalamic area (PSA) is introduced as an alternative target to Vim for tremor. GPi DBS is also effective treatment option for generalized dystonia.

Psychogenic Movement Disorders (PMDs) are a subtype of conversion disorder, classified under somatoform disorders in the DSM. Diagnosis and treatment of PMDs are challenging for both neurologists and psychiatrists. Typical clinical characteristics of these disorders are acute onset, fast progression, movement patterns incongruent with organic movement disorders, distractibility, variability and simultaneous occurrence of various abnormal movements and dysfunctions. The diagnosis of PMDs should not be regarded as a diagnosis of exclusion and electrophysiology is not always helpful. The cause of PMDs is unknown and the underlying brain mechanisms remain uncertain. However, recent functional magnetic resonance imaging studies have demonstrated altered blood flow in conversion disorders that may reflect changes in synaptic activity. Involvement of allied health professionals and psychotherapy continue to be the mainstay of treatment., (Copyright © 2012. Published by Elsevier Masson SAS.)

Object: Wound complications are uncommon following deep brain stimulation (DBS) surgery. However, certain key technical steps can be performed in each procedure to minimize this still troublesome risk. The authors reviewed the incidence and management of all hardware-related wound dehiscences and infections in a large patient series., Methods: All patients undergoing new DBS hardware implantation surgery between 2002 and 2010 by a single surgeon (R.K.S.) were entered into a database after undergoing verification by cross-referencing manufacturer implantation records. All hardware-related complications such as wound dehiscence, erosions, and/or infections were identified, and wound location, time of incidence, and mechanism were categorized. Charts were reviewed to evaluate the success of conservative treatment versus partial or total hardware removal., Results: Seven hundred twenty-eight patients received 1333 new DBS leads and 1218 new implantable pulse generators (IPGs) in a total of 1356 stereotactic procedures for movement disorders. Seventy-eight percent of patients underwent staged lead and IPG implantations. Sixteen patients presented with atraumatic device-related infection and/or dehiscence within 12 months from original implantation; 9 of these patients (1.24%) required additional surgery after antibiotic failure. All 8 patients presenting with cranial wound complications were treated initially by debridement in an attempt to salvage the leads; debridement followed by intravenous antibiotics was only successful in 2 patients presenting with dehiscence alone. One of 2 lead-only removals was successful in infections originating in the cranium; the only IPG-originating infection was treated by partial hardware removal and intravenous antibiotics. Two of 637 IPG replacements resulted in infections within 12 months after revision, requiring either partial or total hardware removal, while 1 dehiscence in this group was treated by debridement alone., Conclusions: In a large series of new DBS hardware implantations, the incidence of postoperative wound dehiscence and/or infections requiring further surgery was 1.24%. Standard practice for all implantations was a short procedural duration, copious povidone-iodine irrigation, and postoperative antibiotic administration. Partial hardware removal should be initially attempted for infection. Debridement alone is successful in treating dehiscence without infection.

Deep brain stimulation (DBS) in the basal ganglia plays an irreplaceable role in the treatment of Parkinson's disease (PD), essential tremor (ET), and some types of dystonia. Electrodes are typically inserted into the subthalamic nucleus (STN), the internal globus pallidus (GPi) and the ventral intermediate nucleus of the thalamus (VIM). Stimulation of the STN is the main objective in PD, GPi in the treatment of dystonia and PD, and VIM stimulation is effective against tremor. In patients indicated for DBS treatment, stimulation eliminates or reduces rigidity (PD), hypokinesia (PD), tremor (ET, PD) and dyskinesias (dystonia, PD). At the Center for Movement Disorders in Prague, DBS has been performed in 127 patients: in 86 patients for PD, 19 patients for dystonia (11 primary generalized dystonia, 5 primary cervical dystonia, 1 segmental craniocervical dystonia), and in 22 patients for tremor (20 ET, 2 other types of tremor). Intraoperatively we have succeeded in eliminating rigidity in 95% of cases of PD, and tremor in 93% of cases of ET. The positive effect of stimulation on dystonia appeared in 95% with a latency of weeks. Transitory intraoperative morbidity occurred in 8%, intracranial hemorrhage in 1%, infectious complications in 6%, and problems with hardware in 7%.

Object: Adverse event (AE) rates for deep brain stimulation (DBS) are variable, due to various methodologies used for identifying, collecting, and reporting AEs. This lack of a prospective, standardized AE collection method is a shortcoming in the advancement of DBS. In this paper the authors disclose the standardized and prospectively recorded AE data from their institution, correlated with clinical outcome and quality of life (QOL) measures., Methods: All patients who underwent operations at the authors' institution for Parkinson disease (PD), essential tremor, dystonia, other tremor, and obsessive-compulsive disorder were included. Complications occurring intraoperatively or within the first 180 days following surgery were recorded, analyzed, and classified as mild, moderate, or severe, regardless of their perceived relationship to the procedure. The presence, frequency, and severity of AEs were compared with the following outcome measurements: postoperative change in the QOL scales (Medical Outcomes Study 36-Item Short-Form Survey, 39-Item PD Questionnaire); motor scales (Tremor Rating Scale, Unified Dystonia Rating Scale, Unified PD Rating Scale); and Patient Global Impression Scale (PGIS)., Results: Two hundred seventy DBS procedures were performed in 198 patients. Three hundred AEs were recorded in 146 (54.1%) of the 270 procedures, and the AEs were recorded in 119 (60.1%) of 198 patients. Of the 198 patients, the maximum severity of AEs was mild in 28 (14.1%), moderate in 35 (17.7%), and severe in 56 (28.3%). Of the 300 AEs, 102 (34.1%) of 299 were mild, 106 (35.5%) were moderate, and 91 (30.4%) were severe. The AEs were classified as probably not stimulation induced in 10 (3.4%) of 297, probably in 44 (14.9%), unclear for 89 (30%), and not applicable to stimulation in 154 (51.9%). Adverse events were also classified as probably related to surgery in 111 (37.2%) of 298, possibly related in 96 (32.2%), and probably not related to surgery in 91 (30.5%). There was no significant difference (p = 0.22) in QOL outcomes among patients who had no AEs compared with those who experienced mild, moderate, or severe AEs. There was no significant difference in QOL outcomes between patients who did not experience an AE compared with those who experienced any AE. There was no significant difference in the mean General PGIS score between patients without an AE versus those with any AE, as well as on the Symptom-Specific PGIS. Motor function outcomes did not vary between patients with or without AEs. For patients with PD with or without AEs, there was no significant difference in preoperative off-medicine Unified PD Rating Scale score and postoperative 6-month on-medication/on-stimulation change scores (p = 0.59). For patients with tremor there were no differences between those with or without AEs on the Tremor Rating Scale for motor function or activities of daily living. Patients with dystonia with and without AEs showed no differences in the Unified Dystonia Rating Scale., Conclusions: Prospectively and systematically recording AEs may result in higher AE rates, but this does not correlate with poorer QOL, motor function, or patient-oriented outcome scores.

Psychogenic movement disorders (PMDs) represent a challenging dilemma for the treating neurologist. The terminology to classify this disorder is confusing and making the diagnosis is difficult. Once the diagnosis has been established, treatment options are limited, and the patient generally does not accept the diagnosis.

Surgical approaches are an important consideration in the management of many movement disorders, particularly for patients refractory to medications. In this article, we review the history, pathophysiology, risks and indications for surgical treatment. Summaries of case studies, case series and clinical trials performed using deep brain stimulation are provided for Parkinson's disease, dystonia, essential tremor and other movement disorders.

The aim of the present study was to review the history, clinical course, treatment, and outcome of movement disorders in children and young people with complex regional pain syndrome (CRPS). Case notes were reviewed retrospectively of children and young people who presented with movement disorders in CRPS to our tertiary paediatric pain service over a period of 13 years. Ten children with CRPS presented with movement disorders (eight females, two males). The age at first presentation with symptoms of CRPS ranged from 8 to 15 years (mean 11 y 2 mo, median 13 y). The most common movement disorder was dystonia (n=8), followed by tremors (n=3) and myoclonus (n=3); two patients had all three movement disorders. The movement disorder affected mainly the lower limb (n=9) with a predilection for the foot (n=7) and was frequently initiated by minor trauma (n=7). Follow-up ranged from 6 months to 14 years. The outcome was variable, with good prognosis in nearly half of the cases: four children experienced complete resolution of symptoms. Two children showed a slight improvement. Four children showed no improvement. Movement disorders in CRPS are under-recognized in children. The management has to be multidisciplinary with an expertise in paediatric pain.

Our knowledge of the clinical, neurophysiological, and genetic aspects of myoclonus-dystonia (M-D) has improved markedly in the recent years. Basic research has provided new insights into the complex dysfunctions involved in the pathogenesis of M-D. On the basis of a comprehensive literature search, this review summarizes current knowledge on M-D, with a focus on recent findings. We also propose modified diagnostic criteria and recommendations for clinical management., (2008 Movement Disorder Society)

Background: Deep brain stimulation is an established symptomatic treatment of movement disorders such as Parkinson's disease, tremor conditions and dystonia when medical treatment fails. We here present a review of indications and results for this treatment. We also present data om the activity related to patients with Parkinson's disease in a representative year and data on implantations performed Rikshospitalet University Hospital in the period 1999-2007., Material and Methods: The manuscript is based on non-systematic searches in PubMed, clinical experience, and internal statistics on implantations and clinical visits carried out at our centre., Results and Interpretation: 243 procedures were performed in our clinic in the period; 187 for Parkinson's disease, 37 for tremor and 19 for dystonia. The vast majority of patients have been implanted with bilateral electrodes, and the targets were the subthalamic nucleus, the thalamus and the internal segment of globus pallidus. Studies have demonstrated that deep brain stimulation is an effective treatment of selected patients with Parkinson's disease, tremors and primary dystonia. Many of these patients have no other efficient treatment options. Patients should be referred for preoperative assessment when symptoms of their movement disorder can no longer be treated sufficiently with medical therapies and when their quality of life is impaired. A broad and careful evaluation of patients' symptoms and findings is important for correct patient selection for this treatment.